Lung Carcinoid Tumors and Paraneoplastic Cushing's Syndrome: Diagnostic and Therapeutic Difficulties - A Case Report


Carcinoid Tumors

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Kamoun H, Ayari A, Rejeb H, Zairi S, Smadhi H, Fekih L, Megdiche ML. Lung Carcinoid Tumors and Paraneoplastic Cushing’s Syndrome: Diagnostic and Therapeutic Difficulties - A Case Report. ATD [Internet]. 2021 Sep. 18 [cited 2021 Oct. 26];2021. Available from:


Introduction. - Paraneoplastic Cushing's syndrome is a rare cause of endogenous hypercorticism. It is caused by ectopic secretion of ACTH by a non-pituitary endocrine tumor. The aetiological biological and radiological data are often inconclusive, which creates a problem of differential diagnosis with Cushing's disease. In addition, these tumors are often small and their location is extremely variable. As a result, the difficulties of localization require the use of specific imaging techniques.
Observation. - We report the observation of a 44-year-old man suffering from diabetes and high blood pressure, presenting a severe and rapidly progressive Cushing syndrome, in connection with a hypercorticism caused by an ectopic ACTH secretion. The thoracic computed tomography performed within the framework of a search for a neoplastic origin objectified a 15 mm nodule isolated at the level of the middle lobe, the scintigraphy with octreotide marked with indium-111 found a significant fixation at the level of the lung nodule. The patient had a middle lobe lobectomy. The outcome was favorable with regression of Cushing's syndrome. Pathological examination was in favor of a typical carcinoid tumor, and the immunohistochemical complement showed tumor cell positivity for ACTH, CD56, chromogranin, and synaptophysin.
Conclusion. - This observation illustrates the dilemma between the need to locate an ectopic ACTH secretion and the control of aggressive and threatening Cushing's syndrome. Early use of the octreotide scintigraphy should be considered if a topographic diagnosis of the causative tumor cannot be done through conventional imaging techniques.


[1] Findling JW, Tyrell JB. Occult ectopic secretion of corticotropin. Arch Intern Med1986;146:929–33.
[2] Orth DN. Cushing’s syndrome. N Engl J Med 1995;332:791–803.
[3] Wajchenberg BL, Mendonca BB, Liberman B, Pereira MA, Carneiro PC, Waka-matsu A, et al. Ectopic adrenocorticotropic hormone syndrome. Endocr Rev1994;15:752–87.
[4] Isidori AM, Kaltsas GA, Grossman AB. Ectopic ACTH syndrome. Front Horm Res2006;3:143–56.
[5] Hage R, Delariviere AB, Seldenrijk CA, Vanden Bosh JR. Update in pulmonarycarcinoid tumors: a review article. Ann Surg Oncol 2003;10:697–704
.[6] Mason AMS, Ratcliffe JG, Buckle RM, Mason S. ACTH secretion by branchialcarcinoid tumors. Clin Endocrinol 1972;1:3–25.
[7] Newell-Price J, Bertagna X, Grossman AB, Nieman LK. Cushing’s syndrome.Lancet 2006;367:1605–17.
[8] Tabarin A. Diagnostic du syndrome de Cushing. Endocrinologie 1999;1:18–36.
[9] Tabarin A, Roger P. Syndrome de Cushing : exploration diagnostique. PresseMed 1994;23:43–8.
[10] Ilias I, Torpy DJ, Pacak K, Mullen N, Wesley RA, Nieman LK. Cushing’s syndromedue to ectopic corticotropin secretion: twenty years experience at the NationalInstitutes of Health. J Clin Endocrinol Metab 2005;90:4955–62.
[11] Malchoff CD, Orth DN, Abboud C, Carney JA, Pairolero PC, Carey RM. Ecto-pic ACTH syndrome caused by a bronchial carcinoid tumor responsive todexamethasone, metyrapone, and corticotropin-releasing factor. Am J Med1988;84:760–4.
[12] Tabarin A, Greselle JF, San-Galli F, Leprat F, Caille JM, Latapie JL, et al. Usefulnessof the corticotropin-releasing hormone test during bilateral inferior petrosalsinus sampling for the diagnosis of Cushing’s disease. J Clin Endocrinol Metab1991;73:53–9.
[13] Nocaudie M. Les scintigraphies des étiologies du syndrome de Cushing. MedNucl 2000;24:421–6.
[14] Pacak K, Ilias I, Chen CC, Carrasquillo JA, Whatley M, Nieman LK.The role of [18F] Fluorodeoxyglucoe positron emission tomography and[111In]- diethylenetriamine- pentaacetate-D-Phe-pentetreotide scintigraphyin the localization of ectopic adrenocortico- tropin-secreting tumors causingCushing’s syndrome. J Clin Endocrinol Metab 2004;89:2214–21.
[15] Scanagatta P, Montresor E, Pergher S, Mainente M, Bonadiman C, Benato C,et al. Cushing’s syndrome induced by bronchopulmonary carcinoid tumours: areview of 98 cases and our experience of two cases. Chir Ital 2004;56:63–70.
[16] Kumar J, Spring M, Carroll PV, Barrington SF, Powrie JK. 18Fluorodeoxyglucosepositron emission tomography in the localization of ectopic ACTH- secretingneuroendocrine tumours. Clin Endocrinol 2006;64:371–4.
[17] Markou A, Manning P, Baya B, Datta SN, Bomnadji JB, Conway GS. [18F]fluoro-2-deoxy-D glucose positron emisssion tomography imaging of thymic carcinoidtumor presenting with recurrent Cushing’s syndrome. Eur J Endocrinol2005;152:521–5.
[18] Gal AA, Kornstein MJ, Cohen C, Duarte IG, Miller JI, Mansour KA. Neuroendocrinetumors of the thymus: a clinicopathological and prognostic study. Ann ThoracSurg 2001;72:1179–82.
[19] Penezic Z, Savic S, Vujovic S, Tatic S, Ercegovac M, Drezgicc M. The ectopic ACTHsyndrome. Srp Arch Celok Lek 2004;132:28–32.
[20] Nieman LK. Medical therapy of Cushing’s disease. Pituitary 2002;5:77–82.[30] Pivonello R, Ferone D, Lambert SW, Colao A. Cabergoline plus lanreotide forectopic Cushing’s syndrome. N Engl J Med 2005;352:2457–8.
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Copyright (c) 2021 Hela Kamoun, Aymen Ayari, Hadhemi Rejeb, Sarra Zairi, Hanene Smadhi, Leila Fekih, Mohamed Lamine Megdiche


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